Feasibility and value of preoperative embolization of a congenital choroid plexus tumour in the premature infant: An illustrative case report with technical details

Abstract

We present a case of a preterm boy (born at 35 weeks of pregnancy) who was delivered urgently by a caesarean section due to placental abruption. The baby was found to have a tense fontanelle leading to imaging that showed a 5.5 cm right intraventricular mass centred in the atrium, hydrocephalus and extensive surrounding vasogenic oedema. The mass was avidly enhancing with a few large associated vessels suggesting high vascularity. The condition of the baby was acutely deteriorating. He had multiple seizures followed by persistent low level of consciousness in the third day of life. His clinical decline prompted decision to proceed with surgical resection and preoperative embolization. A large single feeder (the right posterolateral choroidal artery) was embolized leading to near complete obliteration of the tumour blush. Embolization likely facilitated subsequent surgery the next day. This is based on an estimated blood loss of 250 ml in this 3 kg baby without haemodynamic instability. Gross total resection was achieved with a single surgery and the diagnosis was choroid plexus papilloma. Utilization of this combined approach in this preterm baby had led to survival in this potentially curable disease.

Introduction

Congenital brain tumours are defined as tumours presenting within 2 months of life.¹ They constitute 0.5–1.9% of all paediatric CNS tumours,¹ the majority of which are teratomas. The prognosis is usually guarded except for cases of choroid plexus papilloma, were the prognosis is relatively favourable.¹

A great challenge in surgical management of choroid plexus tumours is the high vascularity of these tumours coupled with the small blood volume in neonates which increases the surgical risk and may limit the degree of surgical resection possible. The perioperative mortality of choroid plexus tumours in paediatric population is estimated to be 12%, mainly from catastrophic blood loss.² Preoperative embolization is an excellent treatment when feasible to minimize that risk and optimize the ability to resect the tumour totally.² However, the technical feasibility and safety of embolization in premature cases of choroid plexus tumours is not well explored in the literature.

We present a premature baby who underwent preoperative embolization of his choroid plexus papilloma with leading to complete resection and survival of this potentially curable disease.

Case presentation

A preterm boy of 35 weeks of gestational age was delivered urgently by caesarean section due to placental abruption. An ultrasound just before delivery showed hydrocephalus. When the baby was delivered, he was fully awake with no motor deficits and normal head circumference; his weight was 3 kg. However, he had bulging fontanelle and lower extremities hyperreflexia. This led to an ultrasound that showed hydrocephalus and intraventricular hyperechogenic mass. The second day of life he developed focal motor seizures, leading to phenobarbital loading, intubations and magnetic resonance imaging (MRI) (Figure 1(a) and (b)). He was found to have a right intraventricular tumour that was avidly enhancing with non-obstructive hydrocephalus. Based on the location of the tumour (epicentre was the atrium) and the degree of enhancement our first differential was choroid plexus tumour, likely a papilloma. The cause of non-obstructive hydrocephalus was likely to be overproduction phenomena. The patient was immediately transferred to our neurosurgical institution for further management (The Montreal Children’s Hospital). Upon arrival to our hospital on third day of life, he was found to be comatose (GCS 7 T). This was despite a good control of his seizures and lack of sedation. He did not have, however, a focal motor deficit or pupillary changes. This prompted us to organize an urgent definitive treatment plan. The clinical condition was managed meanwhile with medical therapy (dexamethasone and mannitol). In the fourth day of life, we performed an angioembolization followed by craniotomy and gross total resection the day after.

Figure 1.

MRI with (a) gad axial and (b) coronal.

Cerebral angiogram findings

Through a right femoral artery approach, a 4-French 65 cm Berestein catheter was advanced and selective catheterization of a right internal carotid artery was performed. The right posterior cerebral artery had foetal configuration, arising directly from the internal carotid artery and was extremely ectatic. The right posterolateral choroidal artery is also extremely ectatic and it supplies multiple branches which are feeding the tumour with a dense neovascular pathological blush which persists throughout the angiogram (Figure 2(a) and (b)). The pathological blush eventually drains into the internal cerebral vein and into the basal vein of Rosenthal.

Figure 2.

Pre-embolization angiogram of right common carotid artery injection (a) AP and (b) lateral (arrow: posterolateral choroidal artery).

Embolization technique

Initially a FAST Tracker 18 then a Baltacci 1.8 microcatheter were advanced into the 4-French Berestein catheter. The Baltacci microcatheter successfully entered the right posterolateral choroidal artery. The angiogram of the right posterolateral choroidal artery demonstrated again the neovascular pathological blush.

Embolization was carried out when an adequate position was achieved using contour emboli 45–150 µm, achieving almost complete devascularization of the tumoural blush. The Baltacci microcatheter was then withdrawn. The post-embolization angiogram of a right common carotid artery documents patency of the right internal carotid artery and its branches. There is significant devascularization with almost complete disappearance of a large neovascular tumoural blush previously described (Figure 3(a) and (b). There were no periprocedural complications and the neurological condition of the patient remained the same (comatose but with symmetrical motor responses to pain).

Figure 3.

Post-embolization angiogram of right common carotid artery injection (a) AP and (b) lateral showing near disappearance of the tumoural blush (arrow: posterolateral choroidal artery).

Clinical course

In the fifth day of life, a gross total resection through posterior temporal corticectomy was feasible in this 3 kg baby. The blood loss was about 250 ml. There was no haemodynamic instability during the procedure, but he required blood products transfusions. An external ventricular drain was inserted at the beginning of the case to control brain swelling and facilitate tumour resection. It was weaned in the following 10 days. Although the patient developed unilateral periventricular haemorrhagic/ischemic changes in postoperative scan (Figure 4(a) and (b)), his level of consciousness recovered. He was extubated, became alert, interactive, feeding orally well and upon his discharge to home he had no visible asymmetry in his exam. However, over the next 8 months it was evident that he had left-sided weakness (involving the arm) mainly, visual field neglect and that he needed a feeding tube. He subsequently needed two operations: 1. a VP shunt; 2. craniotomy and fenestration of loculated quadrigeminal cistern cyst toward the ventricular system. The pathological examination showed grade 1 choroid plexus papilloma. There was no evidence of recurrence or residual on last follow-up (Figure 5).

Figure 4.

MRI with (a) gad axial and (b) coronal. Gross total resection demonstrated in the post-op scan. Note elements of intraventricular and periventricular haemorrhage, as explained in the text.

Figure 5.

MRI with gad axial at last follow-up.

Discussion

The value of preoperative embolization in choroid plexus tumours is well established in the literature. It is emphasized in the paediatric population, as demonstrated by Haliasos et al. who documented reduction in intraoperative blood loss from 224% of total blood volume to 96% and increase in the chances of gross total resection from 41% to 100% when preoperative embolization was utilized.²

There are few reports in the literature about surgical management of choroid plexus tumour in the preterm infant.^3–5 However, only one of them utilized preoperative embolization in a premature baby.⁵

Our case provides further insight in the technical aspect of embolization in the premature baby. We postulate that despite the challenges of accessing and navigating the arterial tree in the preterm baby, the local anatomical vasculature will lend itself favourably for embolization when the congenital tumour is large enough to present clinically. As we can note in our case, the tumour has parasitized the posterior communicating artery (PCOM), leading to an ectatic persistent foetal PCOM. This was valuable for safely navigating toward a single embolizable feeder: the posterolateral choroidal artery.

We suggest that if a similar situation is faced, that the patient to be managed in a centre familiar with angioembolization in newborns and infants.

Declaration of conflicting interests

The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Funding

The authors received no financial support for the research, authorship, and/or publication of this article.